Evidence map›Paper›PMID 40480648›Full record

ArticleRMD open2025

Predictors of persistent active disease and the longitudinal development of organ damage, patient-reported outcomes and autoantibodies in long-term juvenile dermatomyositis.

Simon Girmai Berger, Kristin Schjander Berntsen, Kristine Risum, Henriette Schermacher Marstein, Ivar Sjaastad, Mathis Korseberg Stokke, Helga Sanner

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Article in RMD open, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Simon Girmai BergerOslo University Hospital Institute for Experimental Medical Research, Oslo, Norway.ORCID 0000-0003-4744-3637
Kristin Schjander BerntsenDepartment of Rheumatology, Oslo University Hospital Division of Surgery and Specialized Medicine, Oslo, Oslo, Norway.
Kristine RisumOslo Metropolitan University, Oslo, Norway.ORCID 0000-0001-8210-0360
Henriette Schermacher MarsteinOslo University Hospital Institute for Experimental Medical Research, Oslo, Norway.
Ivar SjaastadOslo University Hospital Institute for Experimental Medical Research, Oslo, Norway.
Mathis Korseberg StokkeOslo University Hospital Institute for Experimental Medical Research, Oslo, Norway.
Helga SannerDepartment of Rheumatology, Oslo University Hospital Division of Surgery and Specialized Medicine, Oslo, Oslo, Norway helga.sanner@medisin.uio.no.ORCID 0000-0001-7226-066X

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveTo quantify longitudinal development of disease activity, organ damage, patient-reported outcomes, and myositis autoantibody profiles in patients with juvenile dermatomyositis (JDM) after long-term follow-up, and to identify predictors for persistent active disease.

methodsForty patients (65% female) diagnosed with JDM were clinically examined at two different time points (visits 1 and 2). We assessed clinically inactive/active disease by the updated PRINTO criteria and the Juvenile DermatoMyositis Activity Index (JDMAI). Organ damage was evaluated by Myositis Damage Index (MDI) and physical function by Childhood Health Assessment Questionnaire (CHAQ/HAQ). Myositis autoantibodies were measured by myositis line immunoassay.

resultsMedian disease duration from symptom onset was 15.1 (2.0-34.6) at visit 1 and 21.7 (7.6-42.7) years at visit 2. At visit 2, active disease (PRINTO) was found in 53%, impaired physical function (CHAQ/HAQ>0) in 40%, organ damage (MDI

conclusionsAfter a median of 21.7 years from symptom onset, the majority of JDM patients still had active disease. Higher organ damage and disease activity at the initial visit predicted persistent active disease at follow-up. These results underscore the chronic nature of JDM, emphasising the need for enhanced early and long-term management strategies to improve patient outcomes.

Indexed as

AutoantibodiesDermatomyositisPatient Reported Outcome MeasuresAdolescentAdultBiomarkersChildDisease ProgressionFemaleFollow-Up StudiesHumansLongitudinal StudiesMalePrognosisSeverity of Illness IndexYoung AdultAutoantibodiesBiomarkersAntirheumatic AgentsAutoantibodiesBiomarkersJuvenile DermatomyositisPatient Reported Outcome Measures

Identifiers

PMID40480648
PMCPMC12161408

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.